Lungenmanifestation der progressiven systemischen Sklerodermie

Progressive systemic scleroderma (PSS) is caused by generalized connective tissue disorder. The most frequent visceral manifestations are pulmonary and oesophageal involvement. Early detection of such visceral manifestations, which are sometimes life-limiting, is of paramount importance in any diagnostic procedures. The sensitivity of the methods differs and depends on the stage of the disease. Modified high-resolution computed tomographic scans (HRCT) were obtained in 28 patients with early forms of PSS and showed pulmonary involvement in 79% and signs of oesophageal involvement in 54%. HRCT is much more sensitive than chest radiography, lung scintigraphy and lung function tests even at an early stage of the disease. Classification of PSS should take account of the clinical type of sclerosis and of immunological and other parameters of prognostic importance.